Growth in North American white children with neurofibromatosis 1 (NF1)

J Med Genet. 2000 Dec;37(12):933-8. doi: 10.1136/jmg.37.12.933.

Abstract

Objective: To analyse the distributions of and generate growth charts for stature and occipitofrontal circumference (OFC) in neurofibromatosis 1 (NF1) patients.

Design: Cross sectional database survey.

Setting: The National Neurofibromatosis Foundation International Database (NFDB) includes clinical information on NF1 patients from 14 participating centres in North America.

Subjects: A total of 569 white, North American, NF1 patients, 55% female and 45% male.

Main outcome measures: Stature and OFC measurements of NF1 patients were compared to age and sex matched population norms using z score standardisation and centile curves.

Results: The distributions of stature and OFC are shifted and unimodal among NF1 patients; 13% of patients have short stature (>/=2 standard deviations below the population mean) and 24% have macrocephaly (OFC >/=2 standard deviations above the population mean).

Conclusions: Alterations of stature and OFC are not limited to NF1 patients with frank short stature or macrocephaly.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Adolescent
  • Body Height
  • Child
  • Child, Preschool
  • Cross-Sectional Studies
  • Female
  • Growth / physiology*
  • Growth Disorders / epidemiology
  • Growth Disorders / physiopathology*
  • Head / growth & development
  • Humans
  • Male
  • Neurofibromatosis 1 / epidemiology
  • Neurofibromatosis 1 / physiopathology*
  • North America / epidemiology
  • Reference Values
  • White People